MRI-Based Circumferential Strain in Boys with Early Duchenne Muscular Dystrophy Cardiomyopathy

Zhan-Qiu Liu; Nyasha G. Maforo; Patrick Magrath; Ashley Prosper; Pierangelo Renella; Nancy Halnon; Holden H. Wu; Daniel B. Ennis

doi:10.3390/diagnostics14232673

Diagnostics (Nov 2024)

MRI-Based Circumferential Strain in Boys with Early Duchenne Muscular Dystrophy Cardiomyopathy

Zhan-Qiu Liu,
Nyasha G. Maforo,
Patrick Magrath,
Ashley Prosper,
Pierangelo Renella,
Nancy Halnon,
Holden H. Wu,
Daniel B. Ennis

Affiliations

Zhan-Qiu Liu: Department of Radiology, Stanford University, Palo Alto, CA 94305, USA
Nyasha G. Maforo: Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, CA 90095, USA
Patrick Magrath: Department of Radiological Sciences, University of California, Los Angeles, CA 90095, USA
Ashley Prosper: Department of Radiological Sciences, University of California, Los Angeles, CA 90095, USA
Pierangelo Renella: Department of Radiological Sciences, University of California, Los Angeles, CA 90095, USA
Nancy Halnon: Department of Pediatrics, University of California, Los Angeles, CA 90095, USA
Holden H. Wu: Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, CA 90095, USA
Daniel B. Ennis: Department of Radiology, Stanford University, Palo Alto, CA 94305, USA

DOI: https://doi.org/10.3390/diagnostics14232673
Journal volume & issue: Vol. 14, no. 23
p. 2673

Abstract

Read online

Background: In boys with Duchenne muscular dystrophy (DMD), cardiomyopathy has become the primary cause of death. Although both positive late gadolinium enhancement (LGE) and reduced left ventricular ejection fraction (LVEF) are late findings in a DMD cohort, LV end-systolic circumferential strain at middle wall (Ecc) serves as a biomarker for detecting early impairment in cardiac function associated with DMD. However, Ecc derived from cine Displacement Encoding with Stimulated Echoes (DENSE) has not been quantified in boys with DMD. We aim to: (1) use cine DENSE to quantify regional Ecc in LGE negative (-) boys with DMD and healthy controls; and (2) compare Ecc with LVEF in terms of differentiating DMD boys with LGE (-) from healthy boys. Methods: 10 LGE (-) boys with DMD and 12 healthy boys were enrolled prospectively in an IRB-approved study for CMR at 3T. Navigator-gated cine DENSE was used to obtain short-axis mid-ventricular data and estimate global and regional Ecc. Group-wise differences were tested via a Wilcoxon rank-sum test. Within-group differences were tested via a Skillings-Mack test followed by pairwise Wilcoxon signed-rank tests. A binomial logistic regression model was adopted to differentiate between DMD boys with LGE (-) and healthy boys. Results: When compared to healthy boys, LGE (-) boys with DMD demonstrated significantly impaired septal Ecc [−0.13 (0.01) vs. −0.16 (0.03), p = 0.019]. In comparison to the Ecc in other segments, both groups of boys exhibited significantly reduced septal Ecc and significantly elevated lateral Ecc. Septal Ecc outperformed LVEF in distinguishing DMD boys with LGE (-) from healthy boys. Conclusions: Reduced septal Ecc may serve as an early indicator of cardiac involvement in LGE (-) DMD boys prior to reduced LVEF and a positive LGE finding.

Published in Diagnostics

ISSN: 2075-4418 (Online)
Publisher: MDPI AG
Country of publisher: Switzerland
LCC subjects: Medicine: Medicine (General)
Website: http://www.mdpi.com/journal/diagnostics

About the journal

Abstract

Keywords